Management of patients with antiphospholipid antibodies: what to do in laboratory scenarios that do not fit the guidelines.

Introduction: Some patients with positive antiphospholipid antibodies (aPL) have not been included in randomized clinical trials or observational registries and, therefore, information on their risk of obstetric or thrombotic recurrence and optimal treatment is scarce.Areas covered: In the present review, the existing evidence regarding the management of two laboratory scenarios not covered by the guidelines is presented: (1) patients with antiphospholipid syndrome (APS) clinical manifestations and aPL positivity not fulfilling APS laboratory criteria, and (2) the possibility of discontinuing anticoagulation in APS patients whose aPL become persistently negative.Expert opinion: Growing evidence suggests a role for low titers and 'non-criteria' aPL, especially in obstetric APS. Treatment is not formally recommended but might be considered according to the individual's risk profile. Regarding the question of whether or not to discontinue anticoagulants after the 'spontaneous' disappearance of aPL, there is no definite answer. Retrospective studies seem to suggest that withdrawal of anticoagulation could be safe in certain patients with APS, especially in those with a first provoked venous thrombosis and whose aPL became persistently negative during follow-up. Still, before the withdrawal can be recommended in routine clinical practice, multicenter and prospective studies are required to validate this hypothesis.

The autoimmune/inflammatory syndrome induced by adjuvants (ASIA)/Shoenfeld's syndrome: descriptive analysis of 300 patients from the international ASIA syndrome registry.

The autoimmune/inflammatory syndrome induced by adjuvants (ASIA) is a recently identified condition in which the exposure to an adjuvant leads to an aberrant autoimmune response. We aimed to summarize the results obtained from the ASIA syndrome registry up to December 2016, in a descriptive analysis of 300 cases of ASIA syndrome, with a focus on the adjuvants, the clinical manifestations, and the relationship with other autoimmune diseases. A Web-based registry, based on a multicenter international study, collected clinical and laboratory data in a form of a questionnaire applied to patients with ASIA syndrome. Experts in the disease validated all cases independently. A comparison study regarding type of adjuvants and differences in clinical and laboratory findings was performed. Three hundred patients were analyzed. The mean age at disease onset was 37 years, and the mean duration of time latency between adjuvant stimuli and development of autoimmune conditions was 16.8 months, ranging between 3 days to 5 years. Arthralgia, myalgia, and chronic fatigue were the most frequently reported symptoms. Eighty-nine percent of patients were also diagnosed with another defined rheumatic/autoimmune condition. The most frequent autoimmune disease related to ASIA syndrome was undifferentiated connective tissue disease (UCTD). ASIA syndrome is associated with a high incidence of UCTD and positive anti-nuclear antibodies (ANA) test. Clinical and laboratory features differ from the type of adjuvant used. These findings may contribute to an increased awareness of ASIA syndrome and help physicians to identify patients at a greater risk of autoimmune diseases following the exposure to vaccines and other adjuvants. The ASIA syndrome registry provides a useful tool to systematize this rare condition.

La investigación sobre tratamientos biológicos en el lupus eritematoso sistémico / Research on biological therapies in systemic lupus erythematosus

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Joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis.

OBJECTIVES: To develop recommendations for the management of adult and paediatric lupus nephritis (LN). METHODS: The available evidence was systematically reviewed using the PubMed database. A modified Delphi method was used to compile questions, elicit expert opinions and reach consensus. RESULTS: Immunosuppressive treatment should be guided by renal biopsy, and aiming for complete renal response (proteinuria <0.5 g/24 h with normal or near-normal renal function). Hydroxychloroquine is recommended for all patients with LN. Because of a more favourable efficacy/toxicity ratio, as initial treatment for patients with class III-IV(A) or (A/C) (±V) LN according to the International Society of Nephrology/Renal Pathology Society 2003 classification, mycophenolic acid (MPA) or low-dose intravenous cyclophosphamide (CY) in combination with glucocorticoids is recommended. In patients with adverse clinical or histological features, CY can be prescribed at higher doses, while azathioprine is an alternative for milder cases. For pure class V LN with nephrotic-range proteinuria, MPA in combination with oral glucocorticoids is recommended as initial treatment. In patients improving after initial treatment, subsequent immunosuppression with MPA or azathioprine is recommended for at least 3 years; in such cases, initial treatment with MPA should be followed by MPA. For MPA or CY failures, switching to the other agent, or to rituximab, is the suggested course of action. In anticipation of pregnancy, patients should be switched to appropriate medications without reducing the intensity of treatment. There is no evidence to suggest that management of LN should differ in children versus adults. CONCLUSIONS: Recommendations for the management of LN were developed using an evidence-based approach followed by expert consensus.

Epidemiology and management of refractory lupus nephritis.

Although the survival of patients with lupus nephritis (LN) has improved considerably in recent years, refractory LN appears in a substantial proportion of patients and, therefore, treatment of LN remains a real challenge today. We will use the term "refractory" LN, for those cases with none or partial response to first-line therapies. In this sense, numerous epidemiological factors, including racial, socioeconomic, histological and serological parameters, may influence treatment response and, therefore, may have an impact on the outcome of renal involvement. Initial conventional therapy will depend somewhat on these epidemiological factors. If this initial therapy fails, fortunately today we have alternative therapies that include the multitarget therapy and the use of biologics. Published evidence about these therapies is presented in this review. Important terms in the management of LN, such as the definition of complete response, partial response, sustained response and renal flare as well as the discrimination of different types of flare, are also discussed here according to the European consensus statement on the terminology used in the management of lupus glomerulonephritis.

[Biological therapies in systemic lupus erythematosus]. / Terapias biolóicas en el lupus eritematoso sistémico.

The immunosuppressive agents used in patients with systemic lupus erythematosus (SLE) have significantly improved prognosis. However, it is necessary to develop more specific immunosuppressive treatments with less toxicity. Better understanding of the mechanisms involved in the loss of tolerance in autoimmune diseases has contributed to the development of potential new treatments called biologic therapies. The targets of these biological therapies are directed toward the B cell depletion, interference in the co-stimulation signals and the blockade of cytokines. Therapies using anti-CD20 monoclonal antibodies have shown satisfactory results especially in patients with SLE refractory to conventional treatment. The biological therapies provide encouraging results that represent a possible option in the treatment of refractory patients as well as a potential therapy in the future management of SLE.

Terapias biológicas en el lupus eritematoso sistémico / Biological therapies in systemic lupus erythematosus

The immunosuppressive agents used in patients with systemic lupus erythematosus (SLE) have signifcantly improved prognosis. However, it is necessary to develop more specifc immunosuppressive treatments with less toxicity. Better understanding of the mechanisms involved in the loss of tolerance in autoimmune diseases has contributed to the development of potential new treatments called biologic therapies. The targets of these biological therapies are directed toward the B cell depletion, interference in the co-stimulation signals and the blockade of cytokines. Therapies using anti-CD20 monoclonal antibodies have shown satisfactory results especially in patients with SLE refractory to conventional treatment. The biological therapies provide encouraging results that represent a possible option in the treatment of refractory patients as well as a potential therapy in the future management of SLE.

Papel de los fármacos biológicos en el tratamiento de las enfermedades autoinmunitarias / Role of biologic therapy in systemic autoimmune diseases

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